摘要
复习了近年来国内外有关早幼粒细胞白血病(promyelocyticleukemia,PML)蛋白研究进展的文献,对PML蛋白的生物学功能进行了综述。近年来的研究表明:所有PML蛋白的亚型均含有RBCC(ringfingerdomain,B-box,coiled-coildomain,总称RBCC)结构域或TRIM结构域(TRIpartitemotif);PML蛋白上的3个SUMO-1(smallubiquitin-relatedmodifier)修饰位点与其在核体(nuclearbodies,NBs)内的定位密切相关,而PML蛋白的NBs内定位对于PML蛋白功能的发挥和NBs的形成至关重要。PML蛋白具有内在的抗病毒活性,具有抑制肿瘤的生长、参与造血祖细胞分化、调节基因转录、诱导细胞凋亡等多种生物学功能。PML基因单倍体活性不足以及PML-RARα融合蛋白的形成不仅是引起APL的基础,也是治疗APL的靶点。
Through reviewing research advances on promyelocytic leukemia(PM L) protein at home and abroad in recent years, the authors summarized the biologic al functions of PML protein in this paper. Recent researches indicated that all PML protein isoforms have RBCC (Ring finger domain, B-box, Coiled-Coil domain , RBCC)or TRIM (TRIpartite motif). There was a close relationship between the t hree SUMO-1 (small ubiquitin-related modifier,SUMO-1)modification site of P ML protein and its location in nuclear bodies (NBs). Moreover, the location of PML protein in NBs is very important for the exertion of the function of PML pr otein and the formation of NBs. PML protein possesses multiple biological functi ons, such as intrinsic antiviral activities, suppressing the growth of tumor, pa rticipating in the differentiation of hemopoietic progenitor, regulating transcr iption of genes, inducing cell apoptosis, and so on. The PML gene haploinsuffici ency and the formation of PML-RARαfusion protein are not only the pathogenesis of APL but also the target of treatment.
出处
《癌症》
SCIE
CAS
CSCD
北大核心
2003年第12期1359-1362,共4页
Chinese Journal of Cancer
关键词
早幼粒细胞白血病
蛋白功能
研究进展
核体
生物学功能
Promyelocytic leukemia (PML) protein
Promyelocytic leukemia-retino ic acid receptor α(PML-RARα)protein
Nuclear body
Biological function
